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2.4.1.225: N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase

This is an abbreviated version!
For detailed information about N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase, go to the full flat file.

Word Map on EC 2.4.1.225

Reaction

UDP-alpha-D-glucuronate
+
N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
=
UDP
+
beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan

Synonyms

diphosphoglucuronate:oligosaccharide uridine glucuronosyltransferase, exostosin, exostosin-1, exostosin-2, Exostosin1, Exostosin2, exotose-2, EXT-1, EXT-2, EXT-3, EXT1, EXT2, EXTL3, extosin, gene EXTL1 glycosyltransferase, gene EXTL2 glycosyltransferase, glucuronic acid/N-acetylglucosamine co-polymerase-1, glucuronosyltransferase, heparan glucuronosyltransferase II, heparan sulfate co-polymerase, heparan sulfate glucuronosyltransferase, heparan sulfate synthesis enzyme, N-acetylglucosaminylproteoglycan beta-1,4-glucuronosyltransferase, RIB-1, RIB-2, sister of tout velu, sister of tout-velu, sotv, tout velu, tout-velu, ttv, UDP-glucuronate:oligosaccharide

ECTree

     2 Transferases
         2.4 Glycosyltransferases
             2.4.1 Hexosyltransferases
                2.4.1.225 N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase

Systematic Name

Systematic Name on EC 2.4.1.225 - N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase

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SYSTEMATIC NAME
IUBMB Comments
UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the human enzyme (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase (EC 2.4.1.224) activity required for the synthesis of the heparan sulfate disaccharide repeats.